Norwood procedure with right ventricle to pulmonary artery conduit: a single-centre 20-year experience

The aim of this study was to evaluate the long-term outcomes of the Norwood procedure with right ventricle-pulmonary artery (RV-PA) conduit for hypoplastic left heart complex.

A retrospective observational study was performed in 136 patients with hypoplastic left heart complex who underwent a Norwood procedure with RV-PA conduit between 1998 and 2017. The probabilities of survival, reintervention and Fontan completion were analysed.

Stage 1 survival was 91.9% (125/136). Reintervention for PA stenosis was needed for 22% and 30% at stages 2 and 3, respectively, while 15% underwent reintervention for aortic arch recoarctation. Among 106 bidirectional Glenn survivors, 93 (68% of the total number of patients) had a Fontan completion, while 4 were not considered to be Fontan candidates. Risk factors for overall mortality included weighing <2.5 kg at the time of the Norwood procedure, intact atrium septum, total anomalous pulmonary vein connection and more than mild atrioventricular regurgitation at the time of the Norwood procedure. Overall survival was 80.9%, 72.3% and 62.8% at 1, 5 and 20 years, respectively.

Probabilities of survival and Fontan completion were acceptable under the current surgical strategy incorporating RV-PA Norwood procedure as the first palliation. Incorporating a strategy to maintain PA growth and ventricular function through the staged repair is of prime importance. Further studies are necessary to observe changes in atrioventricular regurgitation as well as in right ventricular function, in patients who require atrioventricular valve interventions during the staged Fontan completion.